Neonates presenting for specialist neonatal and paediatric surgery range in age and complexity from the extremely premature infant undergoing laparotomy for necrotising enterocolitis to the healthy term neonate undergoing hernia repair. Many patients presenting for repair of congenital abnormalities are born prematurely and/or have congenital cardiac defects, which leads to additional challenges. The most common general surgical conditions presenting in the newborn period are oesophageal atresia (OA) with or without trachea-oesophageal fistula (TOF), congenital diaphragmatic hernia (CDH), intestinal atresias, meconium ileus, malrotation and volvulus, Hirschsprung’s disease, imperforate anus, exomphalos, gastroschisis and necrotising enterocolitis (NEC). Anaesthetic techniques should be tailored to the relevant surgical condition, but most of the key principles of management are similar and require an in-depth knowledge of the anatomical, physiological and pharmacological differences particular to this vulnerable group of patients. In this chapter, we discuss the general considerations for anaesthesia in neonatal surgery and then each specific neonatal condition in relation to their anaesthetic and surgical management.

Total anomalous pulmonary venous return is a rare disorder characterized by the lack of direct connection of the pulmonary veins to the left atrium; instead, the pulmonary veins connect either directly to the right atrium or indirectly via a vein connected to the right atrium. This results in oxygenated blood ultimately draining into the right atrium and mixing with deoxygenated blood, necessitating a right-to-left atrial shunt to survive. Although it can be an isolated defect, in approximately two-thirds of cases total anomalous pulmonary venous return is associated with complex cardiac defects and frequently with heterotaxy syndrome, an embryologic disruption of right and left laterality of the thoracic and abdominal organs. Children with heterotaxy and total anomalous pulmonary venous return have even higher rates of early and late morbidity and mortality. This chapter details the perioperative management of a patient with recurrent pulmonary venous stenosis and intestinal malrotation requiring urgent abdominal surgery and outlines the appropriate management strategies for high-risk patients with this physiology.